Objectives of this present application are to study the biological basis of several neuromuscular disorders as well as to provide an understanding of neuromuscular structure and function which may be ultimately applied to human disease. Our goal is to investigate several selected clinical and basic science areas in considerable depth. Six specific projects have been planned. Investigators in these various laboratories will conduct individual projects and will collaborate in joint enterprises including: biochemical and electrophysiologic studies of the acetylcholine receptor in animals as well as in patients with myasthenia gravis; biochemical and biophysical studies of myotonic dystrophy including ultrastructural studies of erythrocytes from patients with myotonic dystrophy; studies of enzymes possibly associated with ion flux in muscle, nerve and red cells of normal and diseased animals; studies of glycogen storage disease, Reye's syndrome, and rhabdomyolysis and hyperpyrexia after anesthesia; studies of axonal membrane sodium ATPase; and studies of mass distribution and crystalline structure in mammalian myofibrils.